Ventricular Outflow Obstruction Research Articles

Trace elements in patients with aortic valve sclerosis.

Background:Aortic valve sclerosis (AVSc) is defined as the thickening and calcification of aortic valve cusps, in the absence of obstruction of ventricular outflow. AVSc is linked with a clear imbalance in some trace elements.Aims:The objective of this study was to investigate the relationship between AVSc and serum levels of iron (Fe), zinc (Zn), selenium (Se), and copper (Cu). Additionally, this research aimed to explore the clinical significance of human serum zinc, selenium, copper, and iron concentrations as a potential new biomarker for AVSc patients and to clarify the pathophysiological role in individuals at risk of developing AVSc.Patients and methods:The study included 40 subjects with AVSc (25% male and 75% female) who were compared with a healthy control group with the same gender ratio. AVSc was based on comprehensive echocardiographic assessments. Blood samples were taken and Zn and Cu concentrations were determined through the use of atomic absorption spectroscopy. Se was measured using an inductively coupled plasma mass spectrometry device and Fe was measured using a Beckman Coulter instrument.Results:There was a significant difference in the prevalence of diabetes, blood pressure levels, and body mass index between the patients and the healthy subjects (p < 0.05). The differences between the serum Fe, Se, and Cu levels of the AVSc patients and the healthy subjects (p > 0.05) were recorded. The serum Zn of AVSc patients when compared was significantly lower compared with that of the control group (p < 0.01).Conclusion:Patients with AVSc had an imbalance in some of the trace elements in their blood. The patient group’s valves had higher serum Cu levels and lower serum Se, Zn, and Fe concentrations compared with the healthy group’s valves. In the valve patients as compared, AVSc had a high prevalence of obesity, hypertension, and diabetes.

Open Trans-Catheter Mitral Valve Replacement for Mitral Annular Calcification

Mitral valve replacement in patients with severe mitral annular calcification is a high-risk operation with historically poor outcomes. Percutaneous trans-catheter heart valve (THV) implantation is an alternative therapy to the traditional open surgical approach, but carries significant risks including severe paravalvular leak, left ventricular outflow obstruction, and embolization. To address this, alternative treatment modalities have emerged from the growing experience with THV. Here, we describe an open trans-atrial implantation of a balloon expandable THV. Relative to percutaneous therapies, an open approach permits implanting and expanding the valve under direct visual guidance, with anchoring sutures to precisely position the valve. In comparison to traditional surgical approaches, the risk of left circumflex arterial injury and atrioventricular dissociation is reduced as the annular calcification is left alone and no suture lines are run along the annulus. We illustrate this technique using an Edwards Sapien 3 bioprosthetic valve with an Edwards certitude transthoracic delivery system. Mitral valve replacement in patients with severe mitral annular calcification is a high-risk operation with historically poor outcomes. Percutaneous trans-catheter heart valve (THV) implantation is an alternative therapy to the traditional open surgical approach, but carries significant risks including severe paravalvular leak, left ventricular outflow obstruction, and embolization. To address this, alternative treatment modalities have emerged from the growing experience with THV. Here, we describe an open trans-atrial implantation of a balloon expandable THV. Relative to percutaneous therapies, an open approach permits implanting and expanding the valve under direct visual guidance, with anchoring sutures to precisely position the valve. In comparison to traditional surgical approaches, the risk of left circumflex arterial injury and atrioventricular dissociation is reduced as the annular calcification is left alone and no suture lines are run along the annulus. We illustrate this technique using an Edwards Sapien 3 bioprosthetic valve with an Edwards certitude transthoracic delivery system.

Left ventricular outflow obstruction occurs immediately after exercise in patients with non-obstructive hypertrophic cardiomyopathy

We evaluated the hemodynamic and geometric determinants of latent obstruction (LO, trans-left ventricular outflow tract (LVOT) gradient ≥30 mmHg with provocation) in patients with non-obstructive hypertrophic cardiomyopathy (HCMP). A total of 35 patients with non-obstructive HCMP underwent stepwise supine bicycle exercise echocardiography. Trans-LVOT pressure gradients, mitral geometric parameters, left ventricular ejection fractions (LVEF) and left ventricular end-systolic and diastolic dimensions (LVESD, LVEDD) were measured at each stage. The highest peak LVOT pressure gradient predominantly occurred immediately after exercise (n = 32, 91.3%) rather than during peak exercise (n = 3, 8.7%). Significant LO developed in nine patients (25%). No significant differences were found in resting echocardiographic parameters. Compared to the remaining patients, however, patients with LO had longer residual mitral leaflets (defined as residual portions of leaflets after coaptation; 4 ± 4 vs. 13 ± 4 mm, respectively; p = 0.001) and higher resting LVOT pressure gradients (7.4 ± 3.7 vs. 12.9 ± 5.8 mmHg, respectively; p = 0.001). Substantial decreases in mitral annular diameters from peak exercise to recovery after exercise were observed in the LO group, while mitral annular diameters increased after exercise in the non-LO group. In conclusion, the highest peak LVOT pressure gradient predominantly occurred immediately after exercise rather than during peak exercise, regardless of LO. Abrupt decrease of mitral annular diameter immediately after exercise, a longer residual mitral leaflet and a higher resting LVOT pressure gradient at rest might be related to LO.

Half-Turned Truncal Switch Operation for Transposition of The Great Arteries with Left Ventricular Outflow Obstruction

To address various problems with conventional surgical procedures for transposition of the great arteries with left ventricular outflow obstruction, the aortic translocation procedure has been reconsidered as an alternative surgical procedure. We developed another innovative translocation technique, the half-turned truncal switch operation, to make use of various anatomical characteristics. The truncal block involving both semilunar valves is harvested en bloc and anastomosed to the opposite ventricular outflow tract after a half-turn. Temporarily detached coronary arteries are re-anastomosed to the reversed aortic wall defects. The key advantage of this half-turned truncal switch operation is the creation of hemodynamically faultless right and left ventricular outflow tracts including competent valve functions and a coronary circulation ensured by the posteriorly translocated aortic valve, the maximally used autologous pulmonary valve, and anastomosis of the coronary arteries to the confronting aortic wall defects.

Comprehensive assessment of the aortic valve in critically ill patients for the non-cardiologist. Part I: Aortic stenosis of the native valve.

Aortic stenosis (AS) causes left ventricular outflow obstruction. Severe AS has major haemodynamic implications in critically ill patients, in whom increased cardiac output and oxygen delivery are often required. Transthoracic echocardiography (TTE) plays akey role in the AS severity grading. In this review, we will give an overview of how to use the simplified Bernoulli equation to convert the echo Doppler measured velocities (cm s-1) to AS peak and mean gra-dient (mm Hg) and how to calculate the aortic valve area (AVA), using the continuity equation, based on the principle of preservation of flow. TTE allows quantification of compensatory left ventricular (LV) hypertrophy, assessment of LV systolic function, and determination of LV diastolic function and LV loading. Subsequently, the obtained results from the TTE study need to be integrated to establish the AS severity grading. The pitfalls of echocardiographic AS severity assessment are explained, and how to deal with inconsistency between AVA and mean gradient. The contribution of transoesophageal echocardiography, low-dose dobutamine stress echo (in case of low-flow low-gradient AS), echocardiography strain imaging, cardiac magnetic resonance imaging, cardiac multidetector computed tomography and the relatively new concept of Flow Pressure Gradient Classification to the work-up for aortic stenosis is discussed. Finally, the treatment of AS is overviewed. Elective aortic valve replacement is indicated in patients with severe symptomatic AS. In the ICU, afterload reduction by vasodilator therapy and treatment of pulmonary and venous congestion by diuretics could be considered.

Effects of combined myectomy with mitral valve repair on a three-dimensional mitral valve model and left ventricle outflow obstruction in patients with hypertrophic cardiomyopathy

Aim . To assess the effects of combined myectomy with mitral valve repair on a three-dimensional model of the mitral valve in patients with obstructive hypertrophic cardiomyopathy. Methods. 24 patients with obstructive hypertrophic cardiomyopathy and left ventricular outflow obstruction over 50 mm Hg at rest were recruited in a study. Eight patients underwent combined myectomy with mitral valve repair according to the Carpentier method. Seven patients underwent the Alfieri's edge-to-edge repair, and nine patients underwent secondary chordae resection using the Ferrazzi’s technique. Before combined myectomy and two weeks after it, all patients underwent standard transthoracic echocardiography and real-time 3D transesophageal imaging of the mitral valve, followed by quantitative 3D reconstruction of the mitral valve and calculation of the annulus, the leaflets, and the aorto-mitral angle. Results. Despite the selected mitral valve repair technique, we observed a decrease in the LVOT obstruction gradient. There were no differences in the residual obstruction gradient between the selected mitral valve repair technique. Patients with obstructive hypertrophic cardiomyopathy, who underwent combined myectomy and posterior mitral leaflet plasty valve according to the Carpentier approach, reported a correlation of a decrease in the LVOT obstruction with the non-planar angle (r = -0.83; p = 0.040), sphericity index (r = 0.83; p = 0.04) and a decrease in the velocity excursion of the annulus (r = 0.94; p = 0.005). Patients who underwent the Alfieri's edge-to-edge repair demonstrated that the residual LVOT obstruction gradient depended on the annulus height (r = 0.90; p = 0.04) and the ratio of this height to the commissural diameter of the annulus ( r = 0.90; p = 0.04). After secondary chordae resection, a decrease in the LVOT obstruction gradient correlated with the sphericity index (r = 0.77; p = 0.03), the anterolateral-posteromedial annulus diameter (r = -0.72; p = 0.04), the anterior (r = -0.78; p = 0.02) and posterior (r = -0.78; p = 0.02) leaflets, the ratio of the total leaflet length to the anteroposterior diameter of the annulus (r = -0.83; p = 0.01), the area (r = -0.76; p = 0.0з) and the mitral valve tenting height (r = -0.95; p = 0.00). Conclusion .Combined myectomy with mitral valve repair is the method of choice in the treatment of patients with obstructive hypertrophic cardiomyopathy. The comparison of three mitral valve repair techniques did not reveal any differences in the residual LVOT obstruction gradient. However, the Alfieri's edge-to-edge repair may be considered as the most physiological technique to repair dynamic LVOT obstruction.

Low Risk of Hypertrophic Cardiomyopathy With Contemporary Management Strategies Implemented in Non-Referral Regional Community-Based Practices

Major advances in diagnosis and treatment have emerged for hypertrophic cardiomyopathy (HCM), largely in major tertiary referral centers dedicated to this disease. Whether these therapeutic benefits are confined to patients in such highly selected cohorts, or can be implemented effectively in independent regional or community-based populations is not generally appreciated. We assessed management and clinical outcomes in a non-referral HCM center (n = 214 patients) in Eastern Pennsylvania. Over a 6.0 ± 3.2-year follow-up, the HCM-related mortality rate was 0.1% per year attributed to a single disease-related death, in a 49-year-old man with end-stage heart failure, ineligible for heart transplant. Fifteen patients (7%) with prophylactically placed implantable cardioverter-defibrillators (ICDs) experienced appropriate therapy terminating life-threatening ventricular tachyarrhythmias. In 23 other patients (11%; 5%/year), heart failure due to left ventricular outflow obstruction was reversed by surgical septal myectomy (n = 20) or percutaneous alcohol septal ablation (n = 3). This regional HCM cohort was similar to a comparison tertiary center referral population in terms of HCM-mortality: 0.1%/year vs 0.3%/year (p = 0.3) and ICD therapy (31% vs 16% of primary prevention implants), although more frequently with uncomplicated benign clinical course (62% vs 46%; p <0.01). In conclusion, effective contemporary HCM management strategies and outcomes in referral-based HCM centers can be successfully replicated in regional and/or non-referral settings. Therefore, HCM is now a highly treatable disease compatible with normal longevity when assessed in a variety of clinical venues not limited to tertiary centers.

Clinical analysis of 14 infective endocarditis in patients with obstructive hypertrophic cardiomyopathy

Objective: This observational study was aimed to analyze the clinical characteristics of infective endocarditis (IE) in patients with hypertrophic cardiomyopathy (HCM). Methods: A total of 668 patients with IE, and 7 427 patients with HCM were treated in Fuwai Hospital from August 2006 to December 2018. Among them, 14 patients were diagnosed with HCM and IE. The clinical characteristics of these patients including clinical manifestations, pathogen distribution, echocardiography features, in-hospital treatment and outcomes were analyzed retrospectively. Results: The proportion of HCM patients with IE was 0.19%,with the estimated incidence of 0.15/1 000 person-years in HCM patients. Of the 14 patients, 11 patients were male. The most common clinical manifestations were fever and heart murmur, and the main complications were heart failure (12/14) and bacterial embolism (8/14). There were 8 cases (8/14) with positive blood culture, and all causative bacteria were gram positive coccus, in which 5/8 were Streptococcus. The median interventricular septum thickness was (21.2±2.7) mm, and left ventricular outflow obstruction was severe based on echocardiography (Echo) examination. The Echo showed that vegetation was found in all 14 patients and most of the vegetation attached at the anterior leaflet of mitral valve (12/14). The proportions of patients with circulatory embolism (8/14) and valve lesions (12/14) were relatively high. Most cases (10/14) were cured, especially those underwent cardiac surgery (8 cases). The rest 4 cases died with 2 in hospital and 2 after auto-discharge. Conclusions: HCM patients complicated with IE are rare. Septic embolization and valve lesions are common in these patients. IE patients with HCM might have a poor prognosis compared to those without HCM and should receive cardiac surgery as early as possible.

Seria niefortunnych zdarzeń — przewlekła okluzja tętnicy wieńcowej, migotanie przedsionków i udar niedokrwienny. Czy optymalna antykoagulacja jest w tym przypadku możliwa?

Successful chronic total occlusion (CTO) percutaneous coronary intervention (PCI) has been related to discordant benefits concerning clinical and quality life measures. We present a patient previously diagnosed with hypertrophic cardiomyopathy with left ventricular outflow obstruction referred for a CTO of the left anterior descending artery PCI. Crossing via left anterior descending artery was complicated by perforation resulting in haemopericardium (no evidence of tamponade; the patient remained hemodynamically stable). On subsequent days the patient presented with two asymptomatic paroxysmal atrial fibrilation (AF) episodes ( de novo ). In this patient’s case, the stroke’s risk factors did not mandate anticoagulation due to haemopericardium and arrhythmia duration of < 48 hours. Twenty-six hours after the second AF episode, the patient suffered a large ischemic stroke in the territory supplied by the right middle cerebral artery. Despite mechanical thrombectomy (MT), the patient died. Even though CTO PCI is an appealing choice in patients with complex coronary artery disease and chronic total occlusion, the risk still should not be underestimated. Another issue is assessing the thromboembolic risk associated with acute cardioversion of patients with AF estimated to be of < 48 hours duration — it is usually considered low. However, there is increasing evidence that short runs of AF confer a significant risk of stroke. Moreover, it was shown that, among patients undergoing MT for acute ischemic stroke, women had worse outcomes at 90 days.

Abstract 13127: Cardiac Metastasis of Lingual Squamous Cell Carcinoma, A Systematic Review of Presentation, Management, and Outcomes

Introduction: Cardiac metastasis of lingual squamous cell carcinoma (LSCC) describes a rare oncologic progression of oral disease currently ill-defined by medical literature. In this systematic review, we aim to characterize the presentation, management, and outcomes associated with LSCC cardiac metastases. Methods: A comprehensive search of PubMed, Embase, and Cochrane Library databases was performed from inception until December 2019. Inclusion criteria included a histologically diagnosed LSCC, definitive location of cardiac metastasis, and Oxford evidence level of 4. Duplicate, non-English, animal studies, and non-original research were excluded. Patient demographics, presenting symptomology, imaging findings, interventions, and outcomes were collected and analyzed. Results: Twenty-seven articles containing 31 cases met the outlined inclusion criteria. The average age of patient presentation was 55.6 years, ranging from 23-77 years. Average time from primary cancer diagnosis to identification of cardiac metastasis was 2.2 years, varying from 0.2-11.0 years. Shortness of breath (29.0%) and chest pain (29.0%) were the most common presenting symptoms, while effusion (21.2%) and right ventricular outflow obstruction (18.2%) were the predominant echocardiogram findings. Right sided metastases (76.7%) were identified more commonly than left sided metastases (53.3%) and septal metastases (36.7%). Cardiac metastases most commonly presented within the right ventricle (56.7%), followed by the left ventricle (43.3%) (Figure 1). Surgical resection (80.6%) and radiotherapy (74.2%) were the most frequently utilized interventions. No patients obtained remission with treatment and all patients expired within one year of metastatic diagnosis. Conclusions: Cardiac metastases of LSCC demonstrate ventricular predominance and suggest a poor prognosis. Patients may benefit from palliative measures, rather than invasive intervention.

Impaired left atrial stiffness in patients with corrected congenital left ventricular outflow obstructions.

We examined the left atrial stiffness index (LA Stiff) on echocardiography and its determinants in adults and adolescents with repaired congenital left ventricular outflow obstructions (c-LVOOs), including isolated subaortic stenosis (SAS), stenotic bicuspid aortic valve (BAV), and aortic coarctation (CoA). Seventy-two patients (43 males, age: 31 ± 15 years) with repaired c-LVOOs (SAS: n = 12; BAV: n = 27; CoA: n = 33) were compared to 72 age- and sex-matched controls. 2D strain imaging was performed to assess left ventricular (LV) and LA function, including peak positive longitudinal LA strain (LAS), late diastolic LA strain (LDS), and LV global longitudinal strain (GLS). The (E/Ea)/LAS ratio was used to calculate LA Stiff. LA Stiff was significantly higher in the c-LVOO group than in the control group. Among the c-LVOO patients, the CoA group had the lowest GLS and the highest LA Stiff; no significant differences were found with respect to sex, hypertension history, smoking status, or repeated repair among c-LVOO subtypes. Multivariable regression analysis with the variables "BMI" and "c-LVOO subtype" revealed that BMI and c-LVOO subtypes were independently associated with LA Stiff (b = 0.290, P = .009 and b = 0.353, P = .002, respectively). We documented abnormal LA Stiff values in adults and adolescents after c-LVOO repair. Patients with CoA demonstrated the most impaired LA Stiff values. Overweight may contribute to worse LA Stiff values. Further studies are required to determine the prognostic implications of LA Stiff in patients with repaired c-LVOOs.

Prognostic impact of atrial fibrillation in patients with hypertrophic cardiomyopathy in a community-based Japanese cohort: results from Kochi RYOMA study

Abstract Background The prognostic impact of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) is not fully elucidated. Purpose The aim of this study was to examine the prevalence and prognostic impact of AF in a prospectively assembled community-based HCM patient cohort in an aged Japanese community. Methods In 2004, we established a cardiomyopathy registration network in Kochi Prefecture, Japan, consisting of 9 hospitals, and finally 293 patients with HCM were followed. Results The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were men. 86 patients (29%) showed AF. During follow-up period of 6.1±3.2 years, 44 patients died. In those patients, HCM-related deaths occurred in 23 patients with an annual mortality rate of 1.3%. Regarding HCM-related adverse events including HCM-related deaths, appropriate ICD discharge, heart failure admission and hospitalization for embolic events, a total of 77 cardiovascular events in 70 patients occurred. Multivariate analysis revealed that presence of AF, left ventricular (LV) outflow obstruction, NYHA functional class III, and lower LV fractional shortening at registration were significant predictors of these adverse events. During the follow-up period, additional 31 patients (11%) developed new-onset AF. Importantly, the incidence of HCM-related adverse events was significantly higher in patients with new AF observed from its onset compared with those with AF at registration (log-rank p=0.029) (Figure 1). Conclusions In an unselected HCM registry in an aged Japanese community, presence of AF, particularly new-onset AF, was associated with unfavorable clinical outcomes. AF is not just a marker of the disease stage but an important trigger of HCM-related adverse events. Figure 1 Funding Acknowledgement Type of funding source: None

Aortic root enlargement: When and how.

Aortic valve replacement is the definitive management for severe aortic stenosis. Intraoperatively, an aortic root enlargement (ARE) may be used to facilitate the implementation of a suitably sized prosthetic valve. This is to prevent patient prosthesis mismatch (PPM), a condition that causes a left ventricular outflow obstruction. There are four main techniques that are used to perform ARE, namely, Nicks, Manouguian, Nunez (modified Manouguian), and Kanno-Rastan procedures. They each involve incisions through different anatomical structures and allow a variety of valve sizes to be implanted. Studies prove that ARE effectively reduces the incidence of PPM. In addition, they show that there is no definitive link between ARE and perioperative mortality or other complications. There is a scarcity of literature exploring the comparative outcomes of each surgical technique. Therefore, further research is warranted for these procedures to be compared adequately. This review aims to summarise the available literature surrounding ARE with respect to three main questions. (1) What are the indications for ARE, (2) what surgical techniques exist to facilitate ARE, and (3) are there significant differences in patient outcomes when these surgical techniques are employed?

Hypertrophic Cardiomyopathy in Pregnancy.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac condition and highly heterogeneous. Echocardiography and genetic and clinical screening have led to detection in women of childbearing age. Maternal and fetal outcomes among women with HCM are favorable. Genetic counseling is recommended. Prepregnancy clinical evaluation and risk assessment are paramount in ensuring optimal outcomes. Most women carry moderate risk of morbidity, have clinical evaluations and echocardiography each trimester, and deliver vaginally. Those who are symptomatic or have significant left ventricular outflow obstruction or recurrent arrhythmias prior to pregnancy are at higher risk and should be monitored at least monthly.

Right Ventricular Outflow Obstruction by a Membranous Septal Aneurysm in a Ventricular Septal Defect

Right Ventricular Outflow Obstruction by a Membranous Septal Aneurysm in a Ventricular Septal Defect

Percutaneous closure of moderate to large perimembranous ventricular septal defect in small children using left ventricular mid-cavity approach

Aim & objectiveTo report mid-term follow-up result of transcatheter closure of perimembranous Ventricular septal defect (VSD) in children weighing less than 10 kg using Amplatzer Duct Occlude-I (ADO-I) by left ventricular (LV) mid-cavity approach. Material & methodThis is retrospective review of 35 children weighing less than 10 kg with moderate to large perimembranous VSD who were selected for transcatheter closure of VSD using ADO-I in between October 2016 to September 2018. Mean age was 2.08 ± 0.67 years (mean ± SD) and mean weight was 7.2 ± 1.2 kg (mean ± SD). Procedure was done by crossing the VSD from right ventricular side instead of using the standard approach by forming arterio-venous loop. Average fluoroscopic time was 9.2 ± 2.9 min (mean ± SD) and mean procedure time was 34.1 ± 13.1 min (mean ± SD). Mean follow-up period was 8.7 months (3–12 months) ResultDevice closure was successfully done in 32 patients. There was device induced aortic regurgitation (AR) in one case who was sent for surgery. One child was found to have complete heart block on first post-procedure day requiring permanent pace-maker implantation. During follow up none of them had any residual VSD, rhythm disturbance, AR and left or right ventricular outflow obstruction. ConclusionDevice closure can be successfully done in moderate to large perimembranous VSD using left ventricular mid cavity approach in small children. LV mid-cavity approach has advantages in terms of lesser hemodynamic instability, lesser fluoroscopy and lesser chance of device induced AR than standard technique particularly in smaller children.

Morbidity and resource usage after myectomy- or pacing-treatment in hypertrophic obstructive cardiomyopathy: A case-control study

BackgroundTreatment of left ventricular outflow-obstruction (LVOTO) in hypertrophic obstructive cardiomyopathy (HOCM) by short atrio-ventricular delay pacing has long-term hemodynamic results that are not inferior to myectomy, but publications comparing long-term morbidity following those treatments are lacking. MethodsA search for HOCM-patients attending all ten hospitals in the West Götaland Region, Sweden, from 2002 through 2013, identified 251 patients (42 treated with myectomy, 88 with pacing and 121 conservatively). As the age at procedure was significantly lower in the myectomy-group compared to the pacing-group, morbidity was compared by case-control methodology, matching patients for age, maximal wall thickness and LVOT-gradient. We found 31 pairs who constituted the comparison-groups. Post-intervention median follow-up was 15.4 and 10.4 years in pacing- and myectomy-group, respectively. Post-procedural and long-term complications and re-interventions, length of stay, and cost of hospitalization were documented. ResultsBoth treatments improved New York Heart Association class and LVOT-gradients significantly. There were fewer peri-procedural complications in the pacing-group compared to myectomy-group (3.2% and 35.5% p < 0.001). During follow-up pacemaker was implanted in 35.5% of myectomy-group for atrio-ventricular block, 9.7% peri-operatively, and 25.8% during late-follow-up. Furthermore, the pacing group had a superior freedom from all re-interventions, 90.3% versus 61.3% in myectomy-group (p = 0.003). Pacing patients had a shorter in-hospital stay (median 4 [IQR = 2] days) compared to myectomy 11 [7] days; P < 0.001). The mean cost of hospitalization was 74,000 ± 16,000 SEK for pacing and 310,000 ± 180,000 SEK for myectomy, p < 0.001. ConclusionPacing is a simple and reliable treatment for drug-refractory HOCM-patients with low rate of complications and costs.

Transapical Septal Myectomy for Hypertrophic Cardiomyopathy With Midventricular Obstruction

Midventricular obstruction (MVO) is an uncommon variant of hypertrophic cardiomyopathy (HCM). In patients receiving septal myectomy for HCM, failure to recognize the concurrent MVO in the context of basal septum thickening can lead to inadequate excision and residual gradient. In this report, we detail the operative outcomes of MVO with and without coexistent basal septal hypertrophy. From February 1997 through September 2018, 196 patients underwent midventricular myectomy. Medical records and follow-up databases were reviewed to obtain patient characteristics and perioperative features. At baseline, 156 patients (80%) were in New York Heart Association Functional Classification III/IV. Obstruction was isolated to the midventricle in 80 patients, and 63 (79%) were treated by isolated transapical myectomy. The remaining 116 patients had intraventricular obstruction at both subaortic and midcavity levels; in 108 (93%), a combined transaortic and transapical approach was adopted to achieve complete relief of the obstruction. After septal myectomy, the resting peak instantaneous gradient decreased from a median 48 mm Hg (interquartile range [IQR], 23-77 mm Hg) preoperatively to 8 mm Hg (IQR, 0-19 mm Hg) before hospital dismissal. Median follow-up was 2.9 years (IQR, 0.7-5.0 years), and the estimated 1-, 5-, and 10-year survivals were 99%, 98%, and 90%, respectively. There were no late complications attributable to the transapical incision. Transapical exposure is a safe and effective approach for relief of midventricular obstruction, and hemodynamic results are similar to those achieved by standard myectomy for subaortic obstruction. The technique can be combined with transaortic myectomy for patients with left ventricular outflow obstruction at both levels.

Ruptured Aneurysm of the Sinus of Valsalva Causing Pulmonary Embolism: A Rare Association.

Aneurysms of the sinus of Valsalva are rare. Unruptured sinus of Valsalva aneurysm is usually asymptomatic and rarely presents as right ventricular outflow obstruction, myocardial infarction as a result of coronary artery compression, conduction disturbances, or endocarditis. They have only been reported as the presumed source of embolism in six cases. We report a patient with right sinus of Valsalva rupture to the right atrium and embolization of aneurysm contents to the pulmonary vasculature.

Fourth Ventricular Outflow Obstruction in an Infant with Ileal Atresia and Laryngomalacia: Endoscopic Management

AbstractFourth ventricle outflow obstruction (FVOO) is a rare cause of obstructive hydrocephalus. In this study, we described a case of idiopathic FVOO with ileal atresia and laryngomalacia which was managed with endoscopic third ventriculostomy (ETV) and re-endoscopy. We also described the techniques of fenestration of Liliequist membrane and partial removal of arachnoid membrane over dorsum sella (DS) to prevent closure of fenestration and recurrence of hydrocephalus. The patient was a 4-month-old infant presented with progressively increasing head size, feeding difficulty, respiratory distress, and tense fontanel. The infant had a history of laparotomy for ileal atresia. CT scan showed panventriculomegaly due to FVOO. ETV with fenestration of Liliequist membrane was done on emergency basis. After operation, the patient improved clinically and radiologically. Four weeks later, the patient returned with recurrent hydrocephalus. Endoscopic reoperation showed closure of fenestration in arachnoid membrane (Lilieqiest membrane). Endoscopic refenestration with partial excision of arachnoid on DS was done. The patient again recovered radiologically and clinically till last follow-up. In idiopathic FVOO, ETV with wide fenestration of Liliequist membrane, preferably with partial removal of arachnoid on DS, may be very useful in treating hydrocephalus (HCP) and preventing recurrent HCP even in infants.